Multiple structural abnormalities of the spinal cord and brain have been described in patients with MVSD including cerebral polygyria, (7) diastematomyelia, (8) Arnold-Chiari malformation with thoracolumbar meningomyelocele, (9) thoracolumbar rachischisis5 and hydrocephalus due to aqueductal stenosis, spina bifida cystica in the thoracolumbar region, and diastematomyelia.

In 1987, a 57-year-old woman was hospitalized for gait ataxia, dysarthria, dizziness, and bilateral hypertonus 43 months following a posterior fossa decompression and cervical vertebrae level 1 laminectomy, which included placement of a LYODURA[R] graft to correct an Arnold-Chiari malformation and syringomyelia.

ACC can occur as an isolated condition or in combination with other cerebral abnormalities, including Arnold-Chiari malformation, Dandy-Walker syndrome, Andermann syndrome, schizencephaly (clefts or deep divisions in brain tissue), and holoprosencephaly (failure of the forebrain to divide into lobes.

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